The past 15 years has seen significant advances in the characterization of myositis-specific autoantibodies (MSAs) and their associated phenotypes in patients with dermatomyositis (DM). As more careful studies are performed, it is clear that unique combinations of clinical and pathological phenotypes are associated with each MSA, despite the fact that there is considerable heterogeneity within antibody classes as well as overlap across the groups. Because risk for interstitial lung disease (ILD), internal malignancy, adverse disease trajectory, and, potentially response to therapy differ by DM MSA group, a deeper understanding of MSAs and validation and standardization of assays used for detection are critical for optimizing diagnosis and treatment. Like any test, the diagnostic sensitivity and specificity of assays for various MSAs is not perfect. Currently tests for MSAs are helpful at minimum for a clinician to assess relative risk or contribute to diagnosis and perhaps counsel the appropriate patient about what to expect.With international standardization and larger studies it is likely that more antibody tests will make their way into formal schemata for diagnosis and actionable risk assessment in DM. In this review, we summarize key considerations for interpreting the clinical and pathologic associations with MSA in DM and identify critical gaps in knowledge and practice that will maximize their clinical utility and utility for understanding disease pathogenesis.

Lipid disequilibrium induced by inhibition of long-chain acyl-CoA synthetases impairs ERAD substrate glycan trimming and dislocation independently of its effects on lipid droplet biogenesis. The disruptions in ER proteostasis activate the IRE1 and PERK branches of the unfolded protein response and ultimately induce IRE1-dependent cell death.

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Reconstructing the burned ear presents a significant challenge, even in the hands of an experienced surgeon. No one modality will work in all situations; therefore, a range of options should be weighed after the tissue deficit has been defined. Surgeon preference and availability of local tissue are the most important variables.

We describe a rare case of acute type B dissection causing collapse of a previously placed infrarenal stent-graft, resulting in acute limb ischemia due to left iliac limb thrombosis in a 59year-old male. The patient presented with acute back and abdominal discomfort radiating to his back, bilateral buttock stabbing discomfort, and left> right thigh and calf rest pain. CTA showed a spiral type B dissection with collapse of the proximal portion of the EVAR device and left limb occlusion. Urgent treatment with TEVAR distal to the left subclavian covered the entry tear and redirected the majority of the flow to the true lumen leading to near immediate expansion of the proximal portion of the EVAR device. After surgical femoral control, balloon embolectomy of the occluded iliac limb was performed and the limb re-lined. His lower extremity ischemic symptoms resolved and his abdominal and back pain dissipated. At latest 6 month follow-up, CT-A shows an intact TEVAR stent-graft and a widely patent EVAR stent-graft and the patient has no further abdominal, back, or leg symptoms. Acute type B dissection causing proximal abdominal EVAR collapse is an extremely rare presentation of false lumen pressurization and can be treated similarly to complicated type B dissection with the goal of restoring true lumen patency.